Limbic Encephalitis

Post prepared by Precious Ramirez-Arao, Monmouth Medical Center PGY3

A 60 year-old female was found lethargic lying in a pool of feces by roommate.

EMS was called and was immediately brought to the hospital.

In the emergency department she had a witnessed generalized tonic-clonic seizure.

Her roommate relates she had episodes of confusion and short-term memory loss over the past few weeks.

She remained lethargic over the next 72 hours in the hospital.

48-hour EEG monitoring showed diffuse 2 to 3 Hz delta slowing with periodic lateralized epileptiform discharges emanating from the left frontal temporal region.

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T2 weighted image of the brain showed signal abnormality of the left mesial temporal lobe and the pulvinar with diffusion restriction in the left hippocampus consistent with limbic encephalitis.

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Limbic encephalitis (LE) is a subacute syndrome of seizures, personality change and cognitive dysfunction, typically evolving over days to weeks.

Autoimmune and paraneoplastic forms have been described. The most common neoplasms associated with paraneoplastic LE are lung cancer (usually small cell), thymoma, ovarian or testicular teratoma, breast cancer and Hodgkin lymphoma. The associated autoantibody depends on the tumor type. Lung cancer and thymomas are associated with anti-VGKC while ovarian or testicular teratomas are associated with antiNMDA antibodies.

Neurologic symptoms can precede oncologic diagnosis for several months to years and initial CT scans are typically unrevealing.

Nevertheless, prompt and thorough evaluation for malignancy including PET and CT scan of the chest, abdomen and pelvis should be initiated. Symptomatic treatment includes corticosteroids, plasmapharesis and intravenous immune globulin.

 

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Louis Pasteur’s New Jersey Connection

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We have already blogged about Rabies, and the paranoia invoked by this terrible almost invariably fatal illness.

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Part of the terror is the long incubation period between the bite of an infected animal and the onset of the disease, anywhere from 5d to 2 years later.

Before Louis Pasteur’s investigation and research in the late 1800’s, there was nothing a potentially infected victim could do except wait to develop this terrifying disease.

In the 1880s, Louis Pasteur and others proved that Rabies was an infectious disease that could be transmitted between species by infected saliva and blood.

They had also realized that Rabies was neurotrophic and targeted the spinal cord and brain. They could dissect an infected animal, remove the spinal cord, and infect other animals by the inoculation of this material.

Pasteur went on to find that strains of the virus become less lethal (“virulent”) when transmitted from dog to monkey or between other species, and that the virulence diminished with each transmission.

He also found that when sections of rabid spinal cord was suspended in dry air the virulence gradually diminished with time.

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This is how Pasteur produced the first attenuated vaccine, and successfully immunized 50 dogs against rabies.

Then on Monday 6 July 1885, Joseph Meister, aged nine, was brought to him from Alsace having been bitten by a rabid dog just 2 days before.

With some reluctance, Pasteur was persuaded by Drs Vulpian and Grancher of the Académie de Médecine to give Dr Grancher the emulsion from the cord of a rabbit that had died of rabies on 21 June and kept in dry air for 15 days, to innoculate the child.

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The boy was then given 13 further inoculations with samples form progressively fresher (more virulent) infected spinal cord over 10 days.  Pasteur waited every day for news of the boy from his doctos.  Finally when the boy had shown no signs of hydrophobis after three months, it seemed likely that the innoculations had averted the dreaded disease, and Pasteur announced that the vaccine had worked.

Not long after on the other side of the Atlantic in Newark (New Jersey) four boys had been bitten by a dog suspected to be rabid. A well-known physician, Dr. William O’Gorman had heard of Pasteur’s work with Meister, and recommended that the children be sent to him for treatment:

I have such confidence in the preventive forces of inoculation by mitigated virus that were it my misfortune to be bitten by a rabid dog, I would board the first Atlantic steamer, go straight to Paris and, full of hope, place myself immediately in the hands of Pasteur…. If the parents be poor, I appeal to the medical profession and to the humane of all classes to help send these poor children where there is almost a certainty of prevention and cure. Let us prove to the world that we are intelligent enough to appreciate the advance of science and liberal and humane enough to help those who cannot help themselves..

– New York Herald Tribune, December 4, 1885

This appeal to individuals in the United States, for those who desperately needed this medical treatment, as well as to philanthropists who recognized the global need to implement Pasteur’s new discoveries as standard medical procedure, created an uproar throughout the country.

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The four boys were sent to Paris accompanied by the one mother and a reporter.  Their story became a media sensation. It seemed that the entire nation was following the boys, who finally returned home in January 1886 cured.  The papers announced them as “heroes” are arranged for them to  tour and appear in major American cities.

The international publicity and donations resulting from this and other similar cases led to the foundation of the Pasteur Institute in 1888.  Patients came from all around the world for treatment.

By 1911,  the Philadelphia drug company H. K. Mulford was producing a rabies vaccine kit, using the Pasteur method, that could be shipped directly to doctors and was simple enough that “physicians who have had no previous experience may successfully apply it”.

three ampules of rabies vaccine, doses one, two, and three; 26 syringes with physiological salt solution; 26 needles for the syringes; two metal piston rods and two metal finger rests for the syringes; one two-dram vial of tincture of iodine; two charts for recording cases; one letter of general instructions; two stamped return envelopes; one record-of-treatment blank; and one vial of sterile wires. The treatment at this time had been reduced to only 21 doses to be administered one a day for 21 days

The kit contains three ampules of rabies vaccine, doses one, two, and three; 26 syringes with physiological salt solution; 26 needles for the syringes; two metal piston rods and two metal finger rests for the syringes; one two-dram vial of tincture of iodine; two charts for recording cases; and one letter of general instructions.

Watch the video below to find out more about rabies and post-exposure vaccination, which is 100% effective if administered right away: